{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T03:29:01Z","timestamp":1792985341268,"version":"4.5.0"},"reference-count":157,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2021,4,16]],"date-time":"2021-04-16T00:00:00Z","timestamp":1618531200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI16\/00156, PI19\/00204 and PI17\/0957"],"award-info":[{"award-number":["PI16\/00156, PI19\/00204 and PI17\/0957"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002704","name":"Fundaci\u00f3n Cient\u00edfica Asociaci\u00f3n Espa\u00f1ola Contra el C\u00e1ncer","doi-asserted-by":"publisher","award":["Dr. Piris"],"award-info":[{"award-number":["Dr. Piris"]}],"id":[{"id":"10.13039\/501100002704","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Fundacion luchamos por la vida","award":["LPLV_Vaque"],"award-info":[{"award-number":["LPLV_Vaque"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Primary cutaneous T-cell lymphomas (CTCLs) constitute a heterogeneous group of diseases that affect the skin. Mycosis fungoides (MF) and S\u00e9zary syndrome (SS) account for the majority of these lesions and have recently been the focus of extensive translational research. This review describes and discusses the main pathobiological manifestations of MF\/SS, the molecular and clinical features currently used for diagnosis and staging, and the different therapies already approved or under development. Furthermore, we highlight and discuss the main findings illuminating key molecular mechanisms that can act as drivers for the development and progression of MF\/SS. These seem to make up an orchestrated constellation of genomic and environmental alterations generated around deregulated T-cell receptor (TCR)\/phospholipase C, gamma 1, (PLCG1) and Janus kinase\/ signal transducer and activator of transcription (JAK\/STAT) activities that do indeed provide us with novel opportunities for diagnosis and therapy.<\/jats:p>","DOI":"10.3390\/cancers13081931","type":"journal-article","created":{"date-parts":[[2021,4,16]],"date-time":"2021-04-16T10:20:11Z","timestamp":1618568411000},"page":"1931","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":63,"title":["Mycosis Fungoides and S\u00e9zary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0511-5590","authenticated-orcid":false,"given":"Nuria","family":"Garc\u00eda-D\u00edaz","sequence":"first","affiliation":[{"name":"Molecular Biology Department, Universidad de Cantabria\u2014Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, IDIVAL, 39011 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5839-3634","authenticated-orcid":false,"given":"Miguel \u00c1ngel","family":"Piris","sequence":"additional","affiliation":[{"name":"Department of Pathology, Fundaci\u00f3n Jim\u00e9nez D\u00edaz, CIBERONC, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2985-9639","authenticated-orcid":false,"given":"Pablo Luis","family":"Ortiz-Romero","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Hospital 12 de Octubre, Institute i+12, CIBERONC, Medical School, University Complutense, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3913-2495","authenticated-orcid":false,"given":"Jos\u00e9 Pedro","family":"Vaqu\u00e9","sequence":"additional","affiliation":[{"name":"Molecular Biology Department, Universidad de Cantabria\u2014Instituto de Investigaci\u00f3n Marqu\u00e9s de Valdecilla, IDIVAL, 39011 Santander, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,4,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Dobos, G., de Masson, A., Ram-Wolff, C., Beylot-Barry, M., Pham-Ledard, A., Ortonne, N., Ingen-Housz-Oro, S., Battistella, M., d\u2019Incan, M., and Rouanet, J. (2020). Epidemiological Changes in Cutaneous Lymphomas: An Analysis of 8593 Patients from the French Cutaneous Lymphoma Registry. Br. J. Dermatol.","DOI":"10.1111\/bjd.19644"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"5064","DOI":"10.1182\/blood-2008-10-184168","article-title":"Cutaneous Lymphoma Incidence Patterns in the United States: A Population-Based Study of 3884 Cases","volume":"113","author":"Bradford","year":"2009","journal-title":"Blood"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1016\/j.ad.2018.03.006","article-title":"Registro de Linfomas Cut\u00e1neos Primarios de La AEDV: Primer A\u00f1o de Funcionamiento","volume":"109","author":"Servitje","year":"2018","journal-title":"Actas Dermo-Sifiliogr\u00e1ficas"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1182\/blood-2018-11-881268","article-title":"The 2018 Update of the WHO-EORTC Classification for Primary Cutaneous Lymphomas","volume":"133","author":"Willemze","year":"2019","journal-title":"Blood"},{"key":"ref_5","unstructured":"Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., Thiele, J., and Vardiman, J.W. (2017). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissue."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"752","DOI":"10.3109\/10428194.2012.729831","article-title":"Incidence and Survival Patterns of Cutaneous T-Cell Lymphomas in the United States","volume":"54","author":"Imam","year":"2013","journal-title":"Leuk. Lymphoma"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1111\/bjd.17258","article-title":"The PROCLIPI International Registry of Early-Stage Mycosis Fungoides Identifies Substantial Diagnostic Delay in Most Patients","volume":"181","author":"Scarisbrick","year":"2018","journal-title":"Br. J. Dermatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"S168","DOI":"10.1067\/mjd.2002.107231","article-title":"Invisible Mycosis Fungoides: A Diagnostic Challenge","volume":"47","author":"Pujol","year":"2002","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"5830","DOI":"10.1002\/cncr.27627","article-title":"Time Course, Clinical Pathways, and Long-Term Hazards Risk Trends of Disease Progression in Patients with Classic Mycosis Fungoides: A Multicenter, Retrospective Follow-up Study from the Italian Group of Cutaneous Lymphomas","volume":"118","author":"Quaglino","year":"2012","journal-title":"Cancer"},{"key":"ref_10","first-page":"191","article-title":"Follicular Mycosis Fungoides, a Distinct Disease Entity with or without Associated Follicular Mucinosis: A Clinicopathologic and Follow-up Study of 51 Patients","volume":"138","author":"Scheffer","year":"2002","journal-title":"Arch. Dermatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1001\/jamadermatol.2016.1597","article-title":"Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides","volume":"152","author":"Roach","year":"2016","journal-title":"JAMA Dermatol."},{"key":"ref_12","first-page":"3768","article-title":"WHO-EORTC Classifcation for Cutaneous Lymphomas","volume":"105","author":"Willemze","year":"2005","journal-title":"Hematology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1067\/mjd.2002.118538","article-title":"Update on Erythrodermic Cutaneous T-Cell Lymphoma: Report of the International Society for Cutaneous Lymphomas","volume":"46","author":"Vonderheid","year":"2002","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1111\/j.1600-0560.1997.tb00793.x","article-title":"Cutaneous Histopathology of S\u00e9zary Syndrome: A Study of 41 Cases with a Proven Circulating T-Cell Clone","volume":"24","author":"Trotter","year":"1997","journal-title":"J. Cutan. Pathol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1379","DOI":"10.1001\/archdermatol.2012.2089","article-title":"Differential Expression of Programmed Death-1 (PD-1) in S\u00e9zary Syndrome and Mycosis Fungoides","volume":"148","author":"Jansen","year":"2012","journal-title":"Arch. Dermatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1159\/000096191","article-title":"Expression Pattern of Chemokine Receptors and Chemokine Release in Inflammatory Erythroderma and S\u00e9zary Syndrome","volume":"213","author":"Fierro","year":"2006","journal-title":"Dermatology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.1182\/blood-2005-04-1492","article-title":"Skin Homing of S\u00e9zary Cells Involves SDF-1-CXCR4 Signaling and down-Regulation of CD26\/Dipeptidylpeptidase IV","volume":"107","author":"Narducci","year":"2006","journal-title":"Blood"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1016\/j.jid.2019.07.712","article-title":"Overall Survival in Mycosis Fungoides: A Systematic Review and Meta-Analysis","volume":"140","author":"Mourad","year":"2020","journal-title":"J. Investig. Dermatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1713","DOI":"10.1182\/blood-2007-03-055749","article-title":"Revisions to the Staging and Classification of Mycosis Fungoides and S\u00e9zary Syndrome: A Proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Ca","volume":"110","author":"Olsen","year":"2007","journal-title":"Blood"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/j.clindermatol.2015.05.008","article-title":"Systemic Involvement in Mycosis Fungoides","volume":"33","author":"Burg","year":"2015","journal-title":"Clin. Dermatol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.ejca.2018.01.076","article-title":"Blood Classification and Blood Response Criteria in Mycosis Fungoides and S\u00e9zary Syndrome Using Flow Cytometry: Recommendations from the EORTC Cutaneous Lymphoma Task Force","volume":"93","author":"Scarisbrick","year":"2018","journal-title":"Eur. J. Cancer"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"4730","DOI":"10.1200\/JCO.2009.27.7665","article-title":"Survival Outcomes and Prognostic Factors in Mycosis Fungoides\/S\u00e9zary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas\/European Organisation for Research and Treatment of Cancer Staging Proposal","volume":"28","author":"Agar","year":"2010","journal-title":"J. Clin. Oncol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1097\/PAT.0000000000000166","article-title":"Mycosis Fungoides with Large Cell Transformation: Clinicopathological Features and Prognostic Factors","volume":"46","author":"Pulitzer","year":"2014","journal-title":"Pathology"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"3766","DOI":"10.1200\/JCO.2015.61.7142","article-title":"Cutaneous Lymphoma International Consortium Study of Outcome in Advanced Stages of Mycosis Fungoides and S\u00e9zary Syndrome: Effect of Specific Prognostic Markers on Survival and Development of a Prognostic Model","volume":"33","author":"Scarisbrick","year":"2015","journal-title":"J. Clin. Oncol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1182\/blood-2014-11-611194","article-title":"Whole-Genome Sequencing Reveals Oncogenic Mutations in Mycosis Fungoides","volume":"126","author":"Mcgirt","year":"2015","journal-title":"Blood"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1426","DOI":"10.1038\/ng.3444","article-title":"Genomic Profiling of S\u00e9zary Syndrome Identifies Alterations of Key T Cell Signaling and Differentiation Genes","volume":"47","author":"Wang","year":"2015","journal-title":"Nat. Genet."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/ng.3356","article-title":"Genomic Landscape of Cutaneous T Cell Lymphoma","volume":"47","author":"Choi","year":"2015","journal-title":"Nat. Genet."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1490","DOI":"10.1016\/j.jid.2016.03.024","article-title":"Identification of Gene Mutations and Fusion Genes in Patients with S\u00e9zary Syndrome","volume":"136","author":"Prasad","year":"2016","journal-title":"J. Investig. Dermatol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1038\/nature11071","article-title":"Melanoma Genome Sequencing Reveals Frequent PREX2 Mutations","volume":"485","author":"Berger","year":"2012","journal-title":"Nature"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"3720","DOI":"10.1158\/0008-5472.CAN-15-0702","article-title":"The Distinctive Mutational Spectra of Polyomavirus-Negative Merkel Cell Carcinoma","volume":"75","author":"Harms","year":"2015","journal-title":"Cancer Res."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.jid.2016.08.015","article-title":"Shared Oncogenic Pathways Implicated in Both Virus-Positive and UV-Induced Merkel Cell Carcinomas","volume":"137","author":"Derdak","year":"2017","journal-title":"J. Investig. Dermatol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"2034","DOI":"10.1182\/blood-2013-05-504308","article-title":"PLCG1 Mutations in Cutaneous T-Cell Lymphomas","volume":"123","author":"Varela","year":"2014","journal-title":"Blood"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1002\/mgg3.121","article-title":"Personalized Treatment of Sezary Syndrome by Targeting a Novel Ctla4:Cd28 Fusion","volume":"3","author":"Sekulic","year":"2015","journal-title":"Mol. Genet. Genom. Med."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1038\/ng.3370","article-title":"Genomic Analysis of Mycosis Fungoides and S\u00e9zary Syndrome Identifies Recurrent Alterations in TNFR2","volume":"47","author":"Ungewickell","year":"2015","journal-title":"Nat. Genet."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"8470","DOI":"10.1038\/ncomms9470","article-title":"Genomic Analyses Reveal Recurrent Mutations in Epigenetic Modifiers and the JAK-STAT Pathway in S\u00e9zary Syndrome","volume":"6","author":"Kiel","year":"2015","journal-title":"Nat. Commun."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"e450","DOI":"10.3324\/haematol.2015.132837","article-title":"Mutated JAK Kinases and Deregulated STAT Activity Are Potential Therapeutic Targets in Cutaneous T-Cell Lymphoma","volume":"100","author":"Onaindia","year":"2015","journal-title":"Haematologica"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1038\/ng.3442","article-title":"The Mutational Landscape of Cutaneous T Cell Lymphoma and S\u00e9zary Syndrome","volume":"47","author":"Abate","year":"2015","journal-title":"Nat. Genet."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"3387","DOI":"10.1182\/blood-2016-02-699843","article-title":"Candidate Driver Genes in S\u00e9zary Syndrome: Frequent Perturbations of Genes Involved in Genome Maintenance and DNA Repair","volume":"127","author":"Woollard","year":"2016","journal-title":"Blood"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"39627","DOI":"10.18632\/oncotarget.17383","article-title":"Genetic Rearrangements Result in Altered Gene Expression and Novel Fusion Transcripts in S\u00e9zary Syndrome","volume":"8","author":"Izykowska","year":"2017","journal-title":"Oncotarget"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1430","DOI":"10.1182\/blood-2017-02-768234","article-title":"Genomic Analysis of 220 CTCLs Identifies a Novel Recurrent Gain-of-Function Alteration in RLTPR (p.Q575E)","volume":"130","author":"Park","year":"2017","journal-title":"Blood"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1002\/gcc.22679","article-title":"Genomic Analysis Reveals Recurrent Deletion of JAK-STAT Signaling Inhibitors HNRNPK and SOCS1 in Mycosis Fungoides","volume":"57","author":"Cats","year":"2018","journal-title":"Genes Chromosomes Cancer"},{"key":"ref_42","first-page":"147","article-title":"Advanced-stage Mycosis Fungoides. Role of STAT3, NFKB and NFAT Pathways","volume":"182","author":"Cereceda","year":"2019","journal-title":"Br. J. Dermatol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2489","DOI":"10.1182\/bloodadvances.2020001441","article-title":"Branched Evolution and Genomic Intratumor Heterogeneity in the Pathogenesis of Cutaneous T-Cell Lymphoma","volume":"4","author":"Iyer","year":"2020","journal-title":"Blood Adv."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"2681","DOI":"10.1016\/j.jid.2018.06.176","article-title":"An Integrated Data Resource for Genomic Analysis of Cutaneous T-Cell Lymphoma","volume":"138","author":"Chang","year":"2018","journal-title":"J. Investig. Dermatol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"3288","DOI":"10.1182\/blood-2012-04-423061","article-title":"Transcriptome Sequencing in S\u00e9zary Syndrome Identifies S\u00e9zary Cell and Mycosis Fungoides-Associated IncRNAs and Novel Transcripts","volume":"120","author":"Lee","year":"2012","journal-title":"Blood"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"e1306618","DOI":"10.1080\/2162402X.2017.1306618","article-title":"Gene Expression Analysis in Cutaneous T-Cell Lymphomas (CTCL) Highlights Disease Heterogeneity and Potential Diagnostic and Prognostic Indicators","volume":"6","author":"Litvinov","year":"2017","journal-title":"OncoImmunology"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.1182\/bloodadvances.2018022608","article-title":"Single-Cell Heterogeneity in S\u00e9zary Syndrome","volume":"2","author":"Buus","year":"2018","journal-title":"Blood Adv."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"2996","DOI":"10.1158\/1078-0432.CCR-18-3309","article-title":"Single-Cell Profiling of Cutaneous T-Cell Lymphoma Reveals Underlying Heterogeneity Associated with Disease Progression","volume":"25","author":"Borcherding","year":"2019","journal-title":"Clin. Cancer Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"4443","DOI":"10.1158\/1078-0432.CCR-19-0148","article-title":"Single-Cell Lymphocyte Heterogeneity in Advanced Cutaneous T-Cell Lymphoma Skin Tumors","volume":"25","author":"Gaydosik","year":"2019","journal-title":"Clin. Cancer Res."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1175","DOI":"10.1182\/bloodadvances.2018027482","article-title":"Clonotypic Heterogeneity in Cutaneous T-Cell Lymphoma (Mycosis Fungoides) Revealed by Comprehensive Whole-Exome Sequencing","volume":"3","author":"Iyer","year":"2019","journal-title":"Blood Adv."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1042","DOI":"10.1182\/blood-2002-11-3574","article-title":"Mycosis Fungoides Shows Concurrent Deregulation of Multiple Genes Involved in the TNF Signaling Pathway: An Expression Profile Study","volume":"102","author":"Tracey","year":"2003","journal-title":"Blood"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"eaar5894","DOI":"10.1126\/scitranslmed.aar5894","article-title":"High-Throughput Sequencing of the T Cell Receptor b Gene Identifies Aggressive Early-Stage Mycosis Fungoides","volume":"10","author":"Elco","year":"2018","journal-title":"Sci. Transl. Med."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1146\/annurev-immunol-042617-053429","article-title":"Cell Biology of T Cell Receptor Expression and Regulation","volume":"36","author":"Alcover","year":"2018","journal-title":"Annu. Rev. Immunol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1038\/s41586-019-1537-0","article-title":"Structural Basis of Assembly of the Human T Cell Receptor\u2013CD3 Complex","volume":"573","author":"Dong","year":"2019","journal-title":"Nature"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"5484","DOI":"10.1073\/pnas.88.13.5484","article-title":"Functional Activation of the T-Cell Antigen Receptor Induces Tyrosine Phosphorylation of Phospholipase C-Gamma 1","volume":"88","author":"Weiss","year":"1991","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1038\/nri3403","article-title":"T Cell Receptor Signalling Networks: Branched, Diversified and Bounded","volume":"13","author":"Brownlie","year":"2013","journal-title":"Nat. Rev. Immunol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"2936","DOI":"10.1182\/blood-2009-05-220111","article-title":"Monocytes Promote Tumor Cell Survival in T-Cell Lymphoproliferative Disorders and Are Impaired in Their Ability to Differentiate into Mature Dendritic Cells","volume":"114","author":"Wilcox","year":"2009","journal-title":"Blood"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1002\/ajh.24203","article-title":"A Three-Signal Model of T-Cell Lymphoma Pathogenesis","volume":"91","author":"Wilcox","year":"2016","journal-title":"Am. J. Hematol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1016\/j.jid.2019.07.693","article-title":"Frequent and Persistent PLCG1 Mutations in S\u00e9zary Cells Directly Enhance PLC\u03b31 Activity and Stimulate NF\u03baB, AP-1, and NFAT Signaling","volume":"140","author":"Patel","year":"2020","journal-title":"J. Investig. Dermatol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1513","DOI":"10.1182\/blood-2002-08-2434","article-title":"Amplification and Overexpression of JUNB Is Associated with Primary Cutaneous T-Cell Lymphomas","volume":"101","author":"Mao","year":"2003","journal-title":"Blood"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1111\/j.1600-0560.2007.00924.x","article-title":"A Genomic and Expression Study of AP-1 in Primary Cutaneous T-Cell Lymphoma: Evidence for Dysregulated Expression of JUNB and JUND in MF and SS","volume":"35","author":"Mao","year":"2008","journal-title":"J. Cutan. Pathol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"2314","DOI":"10.1016\/j.molimm.2010.05.287","article-title":"The C-Terminal Region of Human NFATc2 Binds CJun to Synergistically Activate Interleukin-2 Transcription","volume":"47","author":"Nguyen","year":"2010","journal-title":"Mol. Immunol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1002\/stem.160178","article-title":"New Perspectives on PKC\u03b8, a Member of the Novel Subfamily of Protein Kinase C","volume":"16","author":"Meller","year":"1998","journal-title":"Stem. Cells"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1146\/annurev-immunol-041015-055347","article-title":"Protein Kinase C Enzymes in the Hematopoietic and Immune Systems","volume":"34","author":"Altman","year":"2016","journal-title":"Annu. Rev. Immunol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1038\/385083a0","article-title":"Selective Modulation of Protein Kinase C-\u03b8 during T-Cell Activation","volume":"385","author":"Monks","year":"1997","journal-title":"Nature"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1146\/annurev.immunol.20.100301.064807","article-title":"Protein Kinase C\u03b8 in T Cell Activation","volume":"20","author":"Isakov","year":"2002","journal-title":"Annu. Rev. Immunol"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1038\/ni.2120","article-title":"A Motif in the V3 Domain of the Kinase PKC-\u03b8 Determines Its Localization in the Immunological Synapse and Functions in T Cells via Association with CD28","volume":"12","author":"Kong","year":"2011","journal-title":"Nat. Immunol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"197","DOI":"10.3389\/fimmu.2012.00197","article-title":"Regulation of PKC-\u03b8 Function by Phosphorylation in T Cell Receptor Signaling","volume":"3","author":"Wang","year":"2012","journal-title":"Front. Immunol."},{"key":"ref_69","first-page":"3394","article-title":"NF-\u039aB Activation Induced by T Cell Receptor\/CD28 Costimulation Is Mediated by Protein Kinase C-Theta","volume":"97","author":"Coudronniere","year":"2000","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"830","DOI":"10.1038\/ni824","article-title":"A Requirement for CARMA1 in TCR-Induced NF-\u039aB Activation","volume":"3","author":"Wang","year":"2002","journal-title":"Nat. Immunol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1111\/imr.12033","article-title":"NF-\u039aB: Roles and Regulation in Different CD4+ T-Cell Subsets","volume":"252","author":"Oh","year":"2013","journal-title":"Immunol. Rev."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"2964","DOI":"10.1007\/s00018-008-8064-8","article-title":"TLR-4, IL-1R and TNF-R Signaling to NF-\u039aB: Variations on a Common Theme","volume":"65","author":"Verstrepen","year":"2008","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_73","first-page":"1","article-title":"NF-\u039aB Signaling in Inflammation","volume":"2","author":"Liu","year":"2017","journal-title":"Signal. Transduct. Target. Ther."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1016\/j.immuni.2005.09.014","article-title":"Phosphorylation of the CARMA1 Linker Controls NF-\u039aB Activation","volume":"23","author":"Sommer","year":"2005","journal-title":"Immunity"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1007\/s13238-013-2084-3","article-title":"NF-\u039aB and STAT3 Signaling Pathways Collaboratively Link Inflammation to Cancer","volume":"4","author":"Fan","year":"2013","journal-title":"Protein Cell"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1186\/gb-2004-5-12-253","article-title":"The Janus Kinases (Jaks)","volume":"5","author":"Yamaoka","year":"2004","journal-title":"Genome Biol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/bs.ircmb.2016.09.012","article-title":"STAT Transcription Factors in T Cell Control of Health and Disease","volume":"331","author":"Goswami","year":"2017","journal-title":"Int. Rev. Cell Mol. Biol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1002\/jcp.10364","article-title":"STAT Proteins: From Normal Control of Cellular Events to Tumorigenesis","volume":"197","author":"Migliavacca","year":"2003","journal-title":"J. Cell. Physiol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1038\/nrm909","article-title":"STATs: Transcriptional Control and Biological Impact","volume":"3","author":"Levy","year":"2002","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1039\/b606246f","article-title":"Structure, Function, and Regulation of STAT Proteins","volume":"2","author":"Lim","year":"2006","journal-title":"Mol. Biosyst."},{"key":"ref_81","doi-asserted-by":"crossref","unstructured":"Dawson, M.A., Bannister, A.J., G\u00f6ttgens, B., Foster, S.D., Bartke, T., Green, A.R., and Kouzarides, T. (2009). JAK2 Phosphorylates Histone H3Y41 and Excludes HP1\u03b1 from Chromatin. Nature.","DOI":"10.1038\/nature08448"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"10213","DOI":"10.1073\/pnas.1221243110","article-title":"Unphosphorylated STAT5A Stabilizes Heterochromatin and Suppresses Tumor Growth","volume":"110","author":"Hu","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1038\/ni.3691","article-title":"Mechanisms and Consequences of Jak-STAT Signaling in the Immune System","volume":"18","author":"Villarino","year":"2017","journal-title":"Nat. Immunol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1146\/annurev-immunol-110416-120628","article-title":"Disorders of the JAK\/STAT Pathway in T Cell Lymphoma Pathogenesis: Implications for Immunotherapy","volume":"35","author":"Waldmann","year":"2017","journal-title":"Annu. Rev. Immunol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1111\/cas.13525","article-title":"Mutations in the Signal Transducer and Activator of Transcription Family of Genes in Cancer","volume":"109","author":"Shahmarvand","year":"2018","journal-title":"Cancer Sci."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1038\/sj.leu.2402093","article-title":"Constitutive STAT3-Activation in Sezary Syndrome: Tyrphostin AG490 Inhibits STAT3-Activation, Interleukin-2 Receptor Expression and Growth of Leukemic Sezary Cells","volume":"15","author":"Eriksen","year":"2001","journal-title":"Leukemia"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1288","DOI":"10.1038\/sj.leu.2403385","article-title":"In Vivo Activation of STAT3 in Cutaneous T-Cell Lymphoma. Evidence for an Antiapoptotic Function of STAT3","volume":"18","author":"Sommer","year":"2004","journal-title":"Leukemia"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1038\/leu.2011.198","article-title":"Constitutive Activation of STAT3 in S\u00e9zary Syndrome Is Independent of SHP-1","volume":"26","author":"McKenzie","year":"2012","journal-title":"Leukemia"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1111\/j.1365-2133.2008.08612.x","article-title":"Prevalence and Treatment of Staphylococcus Aureus Colonization in Patients with Mycosis Fungoides and S\u00e9zary Syndrome","volume":"159","author":"Talpur","year":"2008","journal-title":"Br. J. Dermatol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1182\/blood-2015-08-662353","article-title":"Staphylococcal Enterotoxin A (SEA) Stimulates STAT3 Activation and IL-17 Expression in Cutaneous T-Cell Lymphoma","volume":"127","author":"Krejsgaard","year":"2016","journal-title":"Blood"},{"key":"ref_91","first-page":"1","article-title":"Staphylococcus Aureus Enterotoxins Induce FOXP3 in Neoplastic T Cells in S\u00e9zary Syndrome","volume":"10","author":"Buus","year":"2020","journal-title":"Blood Cancer J."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1182\/blood.V89.1.32.32_32_40","article-title":"Association of Erythrodermic Cutaneous T-Cell Lymphoma, Superantigen- Positive Staphylococcus Aureus, and Oligoclonal T-Cell Receptor V\u03b2 Gene Expansion","volume":"89","author":"Jackow","year":"1997","journal-title":"Blood"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1007\/s00403-019-02015-7","article-title":"Methicillin-Resistant Staphylococcus Aureus (MRSA) Is an Important Pathogen in Erythrodermic Cutaneous T-Cell Lymphoma (CTCL) Patients","volume":"312","author":"Emge","year":"2020","journal-title":"Arch. Dermatol. Res."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1038\/nri3567","article-title":"Human Memory T Cells: Generation, Compartmentalization and Homeostasis","volume":"14","author":"Farber","year":"2014","journal-title":"Nat. Rev. Immunol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1038\/nri2297","article-title":"CCR7 and Its Ligands: Balancing Immunity and Tolerance","volume":"8","author":"Rot","year":"2008","journal-title":"Nat. Rev. Immunol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"23214","DOI":"10.1074\/jbc.M402125200","article-title":"Differential Desensitization, Receptor Phosphorylation,-Arrestin Recruitment, and ERK1\/2 Activation by the Two Endogenous Ligands for the CC Chemokine Receptor 7","volume":"279","author":"Kohout","year":"2004","journal-title":"J. Biol. Chem."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1479","DOI":"10.1007\/s00384-013-1712-y","article-title":"CCR4 Mediates CCL17 (TARC)-Induced Migration of Human Colon Cancer Cells via RhoA\/Rho-Kinase Signaling","volume":"28","author":"Syk","year":"2013","journal-title":"Int. J. Colorectal Dis."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1097\/FJC.0000000000000482","article-title":"G Protein-Coupled Receptor Signaling Through \u03b2-Arrestin-Dependent Mechanisms","volume":"70","author":"Kaur","year":"2017","journal-title":"J. Cardiovasc. Pharmacol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1165\/rcmb.2017-0240OC","article-title":"\u03b2-Arrestin-2-Dependent Signaling Promotes CCR4-Mediated Chemotaxis of Murine T-Helper Type 2 Cells","volume":"58","author":"Lin","year":"2018","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.1189\/jlb.0106035","article-title":"Evidence That Phospholipase C-Dependent, Calcium-Independent Mechanisms Are Required for Directional Migration of T Lymphocytes in Response to the CCR4 Ligands CCL17 and CCL22","volume":"79","author":"Cronshaw","year":"2006","journal-title":"J. Leukoc. Biol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"38781","DOI":"10.1074\/jbc.M110.152173","article-title":"CCR7\/CCL21 Migration on Fibronectin Is Mediated by Phospholipase C\u03b31 and ERK1\/2 in Primary T Lymphocytes","volume":"285","author":"Shannon","year":"2010","journal-title":"J. Biol. Chem."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.ejca.2017.02.027","article-title":"European Organisation for Research and Treatment of Cancer Consensus Recommendations for the Treatment of Mycosis Fungoides\/S\u00e9zary Syndrome\u2014Update 2017","volume":"77","author":"Trautinger","year":"2017","journal-title":"Eur. J. Cancer"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"iv30","DOI":"10.1093\/annonc\/mdy133","article-title":"Primary Cutaneous Lymphomas: ESMO Clinical Practice Guidelines for Diagnosis, Treatment and Follow-Up","volume":"29","author":"Willemze","year":"2018","journal-title":"Ann. Oncol."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"951","DOI":"10.2340\/00015555-3013","article-title":"Treatment of Early Folliculotropic Mycosis Fungoides with Special Focus on Psoralen plus Ultraviolet A","volume":"98","author":"Dalal","year":"2018","journal-title":"Acta Derm. Venereol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1001\/archderm.1989.01670150092016","article-title":"Localized and Disseminated Pagetoid Reticulosis: Diagnostic Immunophenotypical Findings","volume":"125","author":"Mielke","year":"1989","journal-title":"Arch. Dermatol."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1159\/000069967","article-title":"Granulomatous Slack Skin: Treatment with Extensive Surgery and Review of the Literature","volume":"206","author":"Clarijs","year":"2003","journal-title":"Dermatology"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1001\/archderm.134.8.949","article-title":"Topical Corticosteroids for Mycosis Fungoides","volume":"134","author":"Zackheim","year":"1998","journal-title":"Arch. Dermatol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1001\/2013.jamadermatol.541","article-title":"Topical Chemotherapy in Cutaneous T-Cell Lymphoma","volume":"149","author":"Lessin","year":"2013","journal-title":"JAMA Dermatol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.jaad.2015.09.033","article-title":"Guidelines for Phototherapy of Mycosis Fungoides and S\u00e9zary Syndrome: A Consensus Statement of the United States Cutaneous Lymphoma Consortium","volume":"74","author":"Olsen","year":"2016","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1111\/j.1365-4632.2004.02186.x","article-title":"UVB Phototherapy and Skin Cancer Risk: A Review of the Literature","volume":"44","author":"Lee","year":"2005","journal-title":"Int. J. Dermatol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1001\/archderm.141.3.305","article-title":"Long-Term Follow-up of Patients With Early-Stage Cutaneous T-Cell Lymphoma Who Achieved Complete Remission With Psoralen Plus UV-A Monotherapy","volume":"141","author":"Querfeld","year":"2005","journal-title":"Arch. Dermatol."},{"key":"ref_112","first-page":"26","article-title":"Clinical Application of Total Skin Electron Beam (TSEB) Therapy for the Management of T Cell Cutaneous Lymphomas. The Evolving Role of Low Dose (12 Gy) Treatment Schedule","volume":"15","author":"Georgakopoulos","year":"2018","journal-title":"Clin. Transl. Radiat. Oncol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1016\/j.ijrobp.2017.05.052","article-title":"The Results of Low-Dose Total Skin Electron Beam Radiation Therapy (TSEB) in Patients With Mycosis Fungoides From the UK Cutaneous Lymphoma Group","volume":"99","author":"Morris","year":"2017","journal-title":"Int. J. Radiat. Oncol. Biol. Phys."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/S0360-3016(98)00218-1","article-title":"Radiotherapy for Unilesional Mycosis Fungoides","volume":"42","author":"Micaily","year":"1998","journal-title":"Int. J. Radiat. Oncol. Biol. Phys."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1111\/j.1365-2133.1983.tb03991.x","article-title":"Treatment of Cutaneous Lymphoma with Etretinate","volume":"109","author":"Claudy","year":"1983","journal-title":"Br. J. Dermatol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"232","DOI":"10.2340\/0001555567232236","article-title":"Oral Retinoids in Mycosis Fungoides and S\u00e9zary Syndrome: A Comparison of Isotretinoin and Etretinate. A Study from the Scandinavian Mycosis Fungoides Group","volume":"67","author":"Molin","year":"1987","journal-title":"Acta Derm.-Venereol."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"2456","DOI":"10.1200\/JCO.2001.19.9.2456","article-title":"Bexarotene Is Effective and Safe for Treatment of Refractory Advanced-Stage Cutaneous T-Cell Lymphoma: Multinational Phase II\u2013III Trial Results","volume":"19","author":"Duvic","year":"2001","journal-title":"J. Clin. Oncol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1111\/j.1365-2133.2012.11156.x","article-title":"Efficacy and Safety of Bexarotene Combined with Psoralen-Ultraviolet A (PUVA) Compared with PUVA Treatment Alone in Stage IB-IIA Mycosis Fungoides: Final Results from the EORTC Cutaneous Lymphoma Task Force Phase III Randomized Clinical Trial (NCT00056056)","volume":"167","author":"Whittaker","year":"2012","journal-title":"Br. J. Dermatol."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1111\/j.1396-0296.2003.01643.x","article-title":"Interferon in the Treatment of Cutaneous T-Cell Lymphoma","volume":"16","author":"Olsen","year":"2003","journal-title":"Dermatol. Ther."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1200\/JCO.1995.13.1.257","article-title":"Effectiveness of Interferon Alfa-2a Combined with Phototherapy for Mycosis Fungoides and the S\u00e9zary Syndrome","volume":"13","author":"Kuzel","year":"1995","journal-title":"J. Clin. Oncol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"1841","DOI":"10.1111\/jdv.14366","article-title":"Dose-Escalation Study Evaluating Pegylated Interferon Alpha-2a in Patients with Cutaneous T-Cell Lymphoma","volume":"31","author":"Schiller","year":"2017","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"2693","DOI":"10.1111\/jdv.16890","article-title":"European Dermatology Forum\u2014Updated Guidelines on the Use of Extracorporeal Photopheresis 2020\u2014Part 1","volume":"34","author":"Knobler","year":"2020","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/jdv.12311","article-title":"Guidelines on the Use of Extracorporeal Photopheresis","volume":"28","author":"Knobler","year":"2014","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"1784","DOI":"10.1056\/NEJM198912283212603","article-title":"A Randomized Trial Comparing Combination Electron-Beam Radiation and Chemotherapy with Topical Therapy in the Initial Treatment of Mycosis Fungoides","volume":"321","author":"Kaye","year":"1989","journal-title":"New Engl. J. Med."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/S0190-9622(03)01591-3","article-title":"Low-Dose Methotrexate to Treat Mycosis Fungoides: A Retrospective Study in 69 Patients","volume":"49","author":"Zackheim","year":"2003","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"592","DOI":"10.7326\/0003-4819-121-8-199410150-00007","article-title":"Systemic Therapy of Cutaneous T-Cell Lymphomas (Mycosis Fungoides and the Sezary Syndrome)","volume":"121","author":"Bunn","year":"1994","journal-title":"Ann. Intern. Med."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"4091","DOI":"10.1200\/JCO.2011.39.8065","article-title":"Prospective International Multicenter Phase II Trial of Intravenous Pegylated Liposomal Doxorubicin Monochemotherapy in Patients with Stage IIB, IVA, or IVB Advanced Mycosis Fungoides: Final Results from EORTC 21012","volume":"30","author":"Dummer","year":"2012","journal-title":"J. Clin. Oncol."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"4115","DOI":"10.1182\/blood-2011-11-390211","article-title":"Identification of an Active, Well-Tolerated Dose of Pralatrexate in Patients with Relapsed or Refractory Cutaneous T-Cell Lymphoma","volume":"119","author":"Horwitz","year":"2012","journal-title":"Blood"},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1016\/S0190-9622(84)80323-0","article-title":"The Treatment of S\u00e9zary Syndrome","volume":"10","author":"Winkelmann","year":"1984","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"3347","DOI":"10.1200\/JCO.2014.57.5597","article-title":"Long-Term Outcome of Allogeneic Hematopoietic Cell Transplantation for Patients with Mycosis Fungoides and S\u00e9zary Syndrome: A European Society for Blood and Marrow Transplantation Lymphoma Working Party Extended Analysis","volume":"32","author":"Duarte","year":"2014","journal-title":"J. Clin. Oncol."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"2955","DOI":"10.1080\/10428194.2020.1790555","article-title":"Outcomes for Allogeneic Stem Cell Transplantation in Refractory Mycosis Fungoides and Primary Cutaneous Gamma Delta T Cell Lymphomas","volume":"61","author":"Isufi","year":"2020","journal-title":"Leuk. Lymphoma"},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1007\/s40257-018-0380-7","article-title":"Current Status of HDAC Inhibitors in Cutaneous T-Cell Lymphoma","volume":"19","author":"Lopez","year":"2018","journal-title":"Am. J. Clin. Dermatol."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"3109","DOI":"10.1200\/JCO.2006.10.2434","article-title":"Phase IIB Multicenter Trial of Vorinostat in Patients with Persistent, Progressive, or Treatment Refractory Cutaneous t-Cell Lymphoma","volume":"25","author":"Olsen","year":"2007","journal-title":"J. Clin. Oncol."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1080\/10428194.2017.1361022","article-title":"Responses to Romidepsin in Patients with Cutaneous T-Cell Lymphoma and Prior Treatment with Systemic Chemotherapy","volume":"59","author":"Duvic","year":"2018","journal-title":"Leuk. Lymphoma"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"3509","DOI":"10.1158\/1078-0432.CCR-11-3144","article-title":"Romidepsin for Cutaneous T-Cell Lymphoma","volume":"18","author":"Prince","year":"2012","journal-title":"Clin. Cancer Res."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1158\/1078-0432.CCR-12-0290","article-title":"Brentuximab Vedotin","volume":"19","author":"Deng","year":"2013","journal-title":"Clin. Cancer Res."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1097\/PAS.0000000000000571","article-title":"CD30 Expression by B and T Cells: A Frequent Finding in Angioimmunoblastic T-Cell Lymphoma and Peripheral T-Cell Lymphoma-Not Otherwise Specified","volume":"40","author":"Onaindia","year":"2016","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1016\/S0140-6736(17)31266-7","article-title":"Brentuximab Vedotin or Physician\u2019s Choice in CD30-Positive Cutaneous T-Cell Lymphoma (ALCANZA): An International, Open-Label, Randomised, Phase 3, Multicentre Trial","volume":"390","author":"Prince","year":"2017","journal-title":"Lancet"},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"3750","DOI":"10.1200\/JCO.2014.60.3969","article-title":"Phase II Investigator-Initiated Study of Brentuximab Vedotin in Mycosis Fungoides and S\u00e9zary Syndrome with Variable CD30 Expression Level: A Multi-Institution Collaborative Project","volume":"33","author":"Kim","year":"2015","journal-title":"J. Clin. Oncol."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"1520","DOI":"10.1158\/1078-0432.CCR-09-2697","article-title":"Defucosylated Humanized Anti-CCR4 Monoclonal Antibody KW-0761 as a Novel Immunotherapeutic Agent for Adult T-Cell Leukemia\/Lymphoma","volume":"16","author":"Ishii","year":"2010","journal-title":"Clin. Cancer Res. Off. J. Am. Assoc. Cancer Res."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1182\/blood-2009-11-251926","article-title":"S\u00e9zary Syndrome and Mycosis Fungoides Arise from Distinct T-Cell Subsets: A Biologic Rationale for Their Distinct Clinical Behaviors","volume":"116","author":"Campbell","year":"2010","journal-title":"Blood"},{"key":"ref_142","doi-asserted-by":"crossref","unstructured":"Kim, Y.H., Bagot, M., Pinter-Brown, L., Rook, A.H., Porcu, P., Horwitz, S.M., Whittaker, S., Tokura, Y., Vermeer, M.H., and Zinzani, P.L. (2018). Mogamulizumab versus Vorinostat in Previously Treated Cutaneous T-Cell Lymphoma (MAVORIC): An International, Open-Label, Randomised, Controlled Phase 3 Trial. Lancet Oncol., 19.","DOI":"10.1016\/S1470-2045(18)30379-6"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"1847","DOI":"10.1182\/blood-2015-02-625251","article-title":"Mogamulizumab: 2 Birds, 1 Stone","volume":"125","author":"Wilcox","year":"2015","journal-title":"Blood"},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1182\/blood-2018-02-835991","article-title":"CCR4 Mutations Associated with Superior Outcome of Adult T-Cell Leukemia\/Lymphoma under Mogamulizumab Treatment","volume":"132","author":"Sakamoto","year":"2018","journal-title":"Blood"},{"key":"ref_145","doi-asserted-by":"crossref","unstructured":"Wartewig, T., Kurgyis, Z., Keppler, S., Pechloff, K., Hameister, E., \u00d6llinger, R., Maresch, R., Buch, T., Steiger, K., and Winter, C. (2018). Erratum: PD-1 Is a Haploinsufficient Suppressor of T Cell Lymphomagenesis. Nature.","DOI":"10.1038\/nature25142"},{"key":"ref_146","doi-asserted-by":"crossref","unstructured":"Klemke, C.D., Booken, N., Weiss, C., Nicolay, J.P., Goerdt, S., Felcht, M., G\u00e9raud, C., Kempf, W., Assaf, C., and Ortonne, N. (2015). Histopathological and Immunophenotypical Criteria for the Diagnosis of S\u00e9zary Syndrome in Differentiation from Other Erythrodermic Skin Diseases: A European Organisation for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Task Force Study of 9. Br. J. Dermatol.","DOI":"10.1111\/bjd.13832"},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1200\/JCO.19.01056","article-title":"Pembrolizumab in Relapsed and Refractory Mycosis Fungoides and S\u00e9zary Syndrome: A Multicenter Phase II Study","volume":"38","author":"Khodadoust","year":"2020","journal-title":"J. Clin. Oncol."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1016\/S1470-2045(19)30320-1","article-title":"IPH4102, a First-in-Class Anti-KIR3DL2 Monoclonal Antibody, in Patients with Relapsed or Refractory Cutaneous T-Cell Lymphoma: An International, First-in-Human, Open-Label, Phase 1 Trial","volume":"20","author":"Bagot","year":"2019","journal-title":"Lancet Oncol."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"116","DOI":"10.3389\/fmed.2019.00116","article-title":"Novel and Future Therapeutic Drugs for Advanced Mycosis Fungoides and S\u00e9zary Syndrome","volume":"6","author":"Oka","year":"2019","journal-title":"Front. Med."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"888","DOI":"10.1182\/blood-2017-08-802470","article-title":"Activity of the PI3K-\u03b4,g Inhibitor Duvelisib in a Phase 1 Trial and Preclinical Models of T-Cell Lymphoma","volume":"131","author":"Horwitz","year":"2018","journal-title":"Blood"},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1182\/blood-2015-02-629543","article-title":"The MTORC1 Inhibitor Everolimus Has Antitumor Activity in Vitro and Produces Tumor Responses in Patients with Relapsed T-Cell Lymphoma","volume":"126","author":"Witzig","year":"2015","journal-title":"Blood"},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"27S","DOI":"10.1177\/1203475419857668","article-title":"Off-Label Use of Topical Calcineurin Inhibitors in Dermatologic Disorders","volume":"23","author":"Guenther","year":"2019","journal-title":"J. Cutan. Med. Surg."},{"key":"ref_153","doi-asserted-by":"crossref","unstructured":"Lee, E.B., Fleischmann, R., Hall, S., Wilkinson, B., Bradley, J.D., Gruben, D., Koncz, T., Krishnaswami, S., Wallenstein, G.V., and Zang, C. (2014). Tofacitinib versus Methotrexate in Rheumatoid Arthritis. New Engl. J. Med.","DOI":"10.1056\/NEJMoa1310476"},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1016\/j.bjhh.2014.10.001","article-title":"Myeloproliferative Neoplasms and the JAK\/STAT Signaling Pathway: An Overview","volume":"37","year":"2015","journal-title":"Rev. Bras. Hematol. Hemoter."},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1016\/j.jaad.2019.10.016","article-title":"Ruxolitinib Cream for the Treatment of Patients with Alopecia Areata: A 2-Part, Double-Blind, Randomized, Vehicle-Controlled Phase 2 Study","volume":"82","author":"Olsen","year":"2020","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/S0140-6736(20)30609-7","article-title":"Ruxolitinib Cream for Treatment of Vitiligo: A Randomised, Controlled, Phase 2 Trial","volume":"396","author":"Rosmarin","year":"2020","journal-title":"Lancet"},{"key":"ref_157","doi-asserted-by":"crossref","unstructured":"Reilley, M.J., McCoon, P., Cook, C., Lyne, P., Kurzrock, R., Kim, Y., Woessner, R., Younes, A., Nemunaitis, J., and Fowler, N. (2018). STAT3 Antisense Oligonucleotide AZD9150 in a Subset of Patients with Heavily Pretreated Lymphoma: Results of a Phase 1b Trial. J. Immunother. Cancer, 6.","DOI":"10.1186\/s40425-018-0436-5"}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/8\/1931\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T05:48:58Z","timestamp":1760161738000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/8\/1931"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,4,16]]},"references-count":157,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2021,4]]}},"alternative-id":["cancers13081931"],"URL":"https:\/\/doi.org\/10.3390\/cancers13081931","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,4,16]]}}}